Primary biliary cirrhosis

Primary biliary cirrhosis

What is primary biliary cirrhosis?

Primary biliary cirrhosis is the new name for primary biliary cirrhosis, or PBC. Primary biliary cirrhosis is a chronic disease in which the small bile ducts in the liver become injured and inflamed and are eventually destroyed. When there are no bile ducts, bile builds up and causes liver damage. This damage can lead to liver scarring, cirrhosis, and eventually liver failure. Primary biliary cirrhosis is believed to be an autoimmune disease , which means that the immune system is overactive and attacks normal, healthy bile duct cells.

How common is primary biliary cirrhosis?

Researchers estimate that in the United States, about 65 out of every 100,000 women and about 12 out of every 100,000 men have primary biliary cirrhosis.

Who is more likely to get primary biliary cirrhosis?

You are more likely to get primary biliary cirrhosis if you

are a woman

are older than age 35

have a parent or sibling—particularly an identical twin—with the disease

are of Northern European descent

What other health problems do people with primary biliary cirrhosis have?

People with primary biliary cirrhosis may have certain autoimmune diseases, including

autoimmune hepatitis

autoimmune thyroid diseases—conditions in which the immune system attacks the thyroid gland

celiac disease

Raynaud’s disease

Sjögren’s syndrome

scleroderma

People with primary biliary cirrhosis may also have frequent urinary tract infections.

What are the complications of primary biliary cirrhosis?

Common complications of primary biliary cirrhosis include

high blood cholesterol levels

osteoporosis

fat-soluble vitamin deficiencies

These common complications can be prevented and treated.

Primary biliary cirrhosis can also lead to cirrhosis and liver failure.

What are the symptoms of primary biliary cirrhosis?

The most common early symptoms of primary biliary cirrhosis are

feeling tired

itchy skin

Other common early symptoms may include

abdominal pain

nausea

poor appetite

weight loss

arthritis

As the disease gets worse, symptoms may include

weakness

nausea

weight loss

diarrhea

swelling in the legs, ankles, or feet

dark urine

yellowish eyes and skin, called jaundice

raised patches on the skin called xanthomas

Many people have no symptoms when they are first diagnosed with primary biliary cirrhosis. Doctors diagnose up to 6 in 10 people with primary biliary cirrhosis before symptoms begin.2 People with primary biliary cirrhosis and no symptoms are identified through blood tests. Some people do not have symptoms for years after they have been diagnosed with primary biliary cirrhosis.

What causes primary biliary cirrhosis?

The exact cause of primary biliary cirrhosis is unknown. Experts think that a person’s tendency to have an overactive immune system, which may be genetic , and unknown environmental triggers play a role in causing primary biliary cirrhosis. Possible environmental triggers include:

infections

cigarette smoking

exposure to certain chemicals

How do doctors diagnose primary biliary cirrhosis?

Doctors diagnose primary biliary cirrhosis based on your medical and family history, a physical exam, and the results of medical tests.

Your doctor will ask you about your symptoms. He or she will also ask

whether you have a history of certain autoimmune diseases

whether one of your parents or siblings has been diagnosed with primary biliary cirrhosis

about your history of infections and exposure to certain chemicals

Your doctor will examine your body, use a stethoscope to listen to sounds in your abdomen, and tap or press on specific areas of your abdomen. He or she will

look for yellowing of the whites of your eyes and your skin

check to see if your liver and spleen are larger than they should be

check for abdominal tenderness or pain, particularly in the upper right side of your abdomen

What tests do doctors use to diagnose primary biliary cirrhosis?

Blood tests

Your doctor may recommend the following blood tests:

Anti-mitochondrial antibodies (AMA). Anti-mitochondrial antibodies are found in the blood of about 95 percent of people with primary biliary cirrhosis.3

Liver tests. Liver tests can show abnormal liver enzyme levels, which may be a sign of damage in your liver or biliary tract. Higher-than-normal levels of the liver enzyme alkaline phosphatase occur in people with diseases that destroy or block the bile ducts, such as primary biliary cirrhosis.

Your doctor may diagnose primary biliary cirrhosis if you have anti-mitochondrial antibodies and higher-than-normal levels of alkaline phosphatase in your blood, even if you have no other signs or symptoms of the disease.

Cholesterol. People with primary biliary cirrhosis may have higher-than-normal cholesterol levels, which may be a sign that their liver is not working properly.

Imaging tests

Your doctor may use imaging tests such as x-rays and ultrasound to help diagnose primary biliary cirrhosis by ruling out other causes of bile duct damage, such as gallstones, bile duct strictures, and tumors.

Liver biopsy

Your doctor may perform a liver biopsy to

rule out other diseases that may be causing your symptoms

confirm the diagnosis of primary biliary cirrhosis

determine whether the disease is advanced—as shown by the amount of liver scarring or cirrhosis—or very active

How do doctors treat primary biliary cirrhosis?

Doctors treat primary biliary cirrhosis with medicines. Your doctor may prescribe ursodiol (Actigall, Urso). Although ursodiol does not cure primary biliary cirrhosis, it can slow the progression of liver damage. People who respond to ursodiol early in the course of primary biliary cirrhosis can live longer without needing a liver transplant.

If you do not respond to ursodiol, your doctor may prescribe obeticholic acid (Ocaliva). However obeticholic acid does not improve symptoms, and further research is needed to show whether it slows liver disease progression.

Cirrhosis. If primary biliary cirrhosis leads to cirrhosis, doctors can treat the health problems related to cirrhosis with medicines, surgery, and other medical procedures. If cirrhosis leads to liver failure, you may need a liver transplant.

When do doctors consider a liver transplant for primary biliary cirrhosis?

Your doctor will consider a liver transplant when your primary biliary cirrhosis leads to liver failure. Doctors consider liver transplants only after they have ruled out all other treatment options. Talk with your doctor to find out whether a liver transplant is right for you.

What should I eat if I have primary biliary cirrhosis?

You should eat a well-balanced and nutritious diet. Good nutrition is important in all stages of primary biliary cirrhosis to help your liver work properly and manage complications. When primary biliary cirrhosis leads to cirrhosis, you may develop malnutrition because cirrhosis can cause

loss of appetite, which will cause you to eat less

changes in your metabolism

reduced absorption of nutrients

Your doctor can recommend a healthy eating plan that is well balanced and provides enough calories and nutrients. If you have vitamin deficiencies, your doctor may recommend foods that are high in vitamins A, D, E, and K. Your doctor may recommend that you eat foods high in calcium and vitamin D to help prevent osteoporosis .

If you have vitamin deficiencies, your doctor may recommend foods that are high in vitamins A, D, E, and K.

What foods should I avoid eating if I have primary biliary cirrhosis?

You should avoid eating raw shellfish such as oysters, which can have bacteria that may cause severe infections in people with liver disease. Your doctor may recommend that you make healthy food choices and avoid high-salt foods and foods that are high in fat and carbohydrates, especially those with added sugars.

If you have primary biliary cirrhosis, your doctor will recommend that you quit smoking and stop drinking alcohol or, at least, limit your intake to no more than one or two drinks per week. If you have primary biliary cirrhosis and cirrhosis, your doctor will recommend complete avoidance of alcohol.